Anémie hémolytique auto-immune à IgM à large amplitude thermique réfractaire à la corticothérapie associée à une hypogammaglobulinémie profonde : une observation inhabituelle: Warm IgM-mediated autoimmune hemolytic anemia with large recurrent thermal amplitude that is refractory to corticotherapy and associated with deep hypogammaglobinemia: an unusual case

Autoimmune haemolytic anaemia (AIHA) is caused by autoantibodies produced against red blood cells. AIHA encompasses several bio clinical entities. Determining the type of haemolytic anaemia is a crucial diagnostic step that must consider the immunochemical properties of the autoantibody involved as well as the presence or not of an underlying disease. Rituximab is the second line treatment regimen when corticosteroids fail (refractory AIHA) unless the anaemia is associated to a pre-existing hypogammaglobulinaemia. Thus real questions have to be raised, and precisely the one related to Rituximab-induced lymphocytic depletion (lymphopenia). We are reporting the case of a 79 year-old female patient followed up with an IgM AIHA since 2016. The patient was initially treated with corticosteroids at high dose with good outcome in the first year of treatment. A year after being diagnosed and discharged, she was admitted again for anaemia and benefited of biochemical investigations in favour of AIHA. Viral serologies and imaging were unremarkable, but she had a severe hypoglobulinaemia (2G/L) suggestive of common variable immune deficiency despite her age. In brief, it was the case of an isolated and rare IgM AIHA, refractory to corticosteroids associated with severe hypogammaglobulinaemia. Therefore, Rituximab and polyvalent immunoglobulins (Polygam) substitution even in the presence of an asymptomatic hypogammmaglobulinaemia remain the last optional treatment. Finally, with this treatment, she experienced correction of anaemia without major complications. Les anémies hémolytiques auto-immunes (AHAI) sont causées par des auto-anticorps dirigés contre les globules rouges. Elles regroupent plusieurs entités clinicobiologiques. La détermination du type d’anémie hémolytique est une étape cruciale de la démarche diagnostique et doit tenir compte aussi bien des propriétés immunochimiques de l’auto-anticorps en cause que de la présence ou non d’une pathologie sous-jacente. Le traitement par Rituximab en deuxième ligne thérapeutique, en cas d’une AHAI réfractaire à la corticothérapie est indiqué, excepté lorsqu’elle est associée à une hypogammaglobilinémie préexistante suscitant des questions, notamment celle relative à la déplétion lymphocytaire secondaire à cette molécule. Nous rapportons ici, le cas d’une patiente de 79 ans suivie pour Anémie hémolytique auto-immune à IgM à large amplitude thermique depuis 2016. Dans un premier temps, elle avait été mise sous une corticothérapie à des doses élevées. L’évolution au cours de la première année de prise en charge était bonne. Une année après le diagnostic, elle était réadmise pour anémie et une AHAI avait été diagnostiquée. Les sérologies virales et l’imagerie étaient sans particularité mais les tests immunologiques retrouvaient une hypogammaglobilinémie profonde à 2g/L suggérant, un déficit immunitaire commun variable (DICV) malgré son âge. En bref, le diagnostic retenu a été l’anémie hémolytique auto-immune à IgM à large amplitude thermique, réfractaire à la corticothérapie et associée à une hypogammaglobulinémie profonde. Un traitement fait du Rituximab associé à la substitution en immunoglobulines polyvalentes a été essayé. Sous cette dernière attitude thérapeutique, l’évolution a été favorable, avec correction de l’anémie sans complications majeures

Association Syndrome SAPHO et Maladie inflammatoire chronique de l’intestin : une observation clinique inhabituelle: SAPHO syndrome associated with chronic inflammatory bowel disease: an unsual case report

The authors report a clinical case reporting, on the one hand, a chronic inflammatory bowel disease and, on the other hand, synovitis, spondylitis, acne, pustulosis, hyperostosis and osteitis that found in the SAPHO syndrome. The latter, which is a rare form of spondyloarthritis, was first observed in a 31-year-old Congolese patient followed by the Rheumatology unit of the University Hospitals of Kinshasa. Digestive signs included episodes of non-febrile bloody glairo-diarrhoea and rectorragia. Colonoscopy and histopathological examination had diagnosed ulcerative colitis. Les auteurs rapportent un cas clinique associant, d’une part, une maladie inflammatoire chronique intestinale et, d’autre part, des synovites, une spondylite, des acnés, une pustulose, une hyperostose et une ostéite qui entrent dans le cadre du syndrome SAPHO. Ce dernier qui est une forme rare de Spondylarthrite a été observée pour la première fois chez un patient congolais de 31 ans suivi au service de Rhumatologie des Cliniques Universitaires de Kinshasa. Les signes digestifs comprenaient d’épisodes de diarrhées glairo sanguinolentes non fébriles et de rectorragie. La colonoscopie et l’examen histopathologique avaient permis de diagnostiquer une rectocolite ulcérohémorragique

Normative Values of Bone Mineral Content and Bone Mineral Density Assessed by Double X-ray Absorptiometry in Congolese Urban Women

Introduction: The World Health Organization (WHO) have validated dual X-ray absorptiometry as the “gold standard” densitometric technique for assessing Bone Mineral Density (BMD) and Bone Mineral Content (BMC).The definitions of osteopenia and osteoporosis are based on its results Loss of bone mass with ageing or osteoprosis leads to decline of bone strength and fragility fractures. There are racial/ethnic differences in bone mass parameters for populations of different ethnic origins although living in the same environmental. WHO criteria for the diagnosis of osteoporosis and the associated risks of fractures are based on bone parameters assessed by dual x absorptiometry in postmenopausal Caucasian women. Studies have shown the necessity to establish reference data for bone mass measurements for each population according to habit and ethnicity.These data are lacking for Congolese populations. This study aims to establish spine and hip normative values in healthy Congolese women population and to compare them with those for Caucasian, Asian, and others ethnical groups. Materials and methods: 604 bantus women aged from 18 to 92 years were recruited after public media advertising and undergoes DXA of spine and hip. To be included in the study, women must fulfill the conditions of absence of factor affecting bone metabolism. Results: Bone mass parameters shows a growth up to the peak that is reached in the fourth decade followed by a slow decay that causes a loss of nearly 14.2% in BMD and BMC over a period of 20 years and, then a decrease more pronounced towards the sixtieth year (1.5% yearly), higher than among Caucasians Asians and Arabs. We found that the references curves for the lumbar spine and total hip are significantly differences from the Caucasian, Asean or Arab. Conclusion: Spine and hip normatives values of BMD and BMC of Congolese urban women was etablished and were different to those of compared populations

Aspects épidémiologiques et cliniques des manifestations rhumatismales au cours de l’infection à VIH en milieu hospitalier de Kinshasa: Epidemiological and clinical aspects of rheumatic manifestations in HIV patients in Kinshasa. A hospital- based study

Context and objective. Data on articular manifestations of HIV infection in sub-Saharan Africa are scarce. The present study aimed to describe clinical features of rheumatic manifestations in Congolese HIV patients. Methods. A cross-sectional study including HIV patients was conducted from June 1st to September 30th, 2015 in two hospitals of Salvation Army in Kinshasa, DRC. The parameters of interest included musculoskeletal signs, radiographic and laboratory data of each patient. Rheumatic diseases were defined according to classical criteria or physician opinion if criteria were not applicable. Results. Three hundred twenty one HIV patients were recruited, of whom 254 women (79.1%). Their average age was 45.1 ± 9.56 years. The median duration of HIV infection and antiretroviral therapy was 38 months (ranged between 3 and 155 months) and 34 months respectively (ranged between 2 to 137 months). The prevalence of rheumatic complaints in HIV was 27.7%. The following diseases were encountered: osteoarthritis (49.4%), spondyloarthitis (33.7%), soft tissue rheumatisms (25.8%), rheumatoid arthritis (1.1%) and miscellaneous (16.9%). HIV patients with rheumatic symptoms showed no difference to those without rheumatic symptoms concerning the WHO classification system for HIV infection, the number of T-CD4 lymphocytes and the duration of HIV infection. A disease or a treatment duration between 12 and 60 months was associated to a high frequency of spondylarthritis and soft tissue rheumatisms. The risk of soft tissue rheumatisms occurrence was fourfold highest in patients with low T-CD4 lymphocytes. Conclusion. Roughly, one third of HIV patients exhibits various rheumatic diseases, mainly osteoarthritis, spondylarthritis and soft tissue rheumatism. Contexte et objectif. Les données sur l’atteinte articulaire dans l’infection à VIH en Afrique subsaharienne l’épicentre sont fragmentaires. La présente étude a décrit les aspects épidémiologiques des manifestations rhumatismales, au cours de l’infection à VIH/SIDA ; en milieu hospitalier de Kinshasa. Méthodes. Etude transversale descriptive et analytique incluant les personnes vivant avec VIH menée du 1er juin au 30 septembre 2015 dans deux formations médicales de l’Armée du Salut. Les paramètres d’intérêt étaient : démographiques, cliniques (symptômes musculosquelettiques), radiographiques et biologiques. Le diagnostic des pathologies rhumatismales a été posé sur base des critères classiques ou le cas échéant, l’opinion du rhumatologue pour les maladies sans critères diagnostiques consensuels. Résultats. Trois cent vingt et un PVV ont été examinés, dont 254 femmes (79,1%). L’âge moyen des patients était de 45,1 ± 9,6 ans. La durée médiane de l’infection à VIH et du traitement antirétroviral étaient respectivement de 38 mois [extrêmes 3 et 155 mois) et 34 mois (extrêmes 2 et 137 mois). La fréquence des manifestations rhumatismales était 27,7%. Les pathologies rhumatismales rencontrées comprenaient: l’arthrose (49,4%), les spondylarthrites (33,7%), les pathologies abarticulaires (25,8%), la polyarthrite rhumatoïde (1,1%) et les autres rhumatismes (16,9%). Les caractéristiques de l’infection à VIH (stade de l’OMS, taux des LT CD4, durée de la maladie, durée du traitement) étaient similaires entre les rhumatisants et les non rhumatisants. Une maladie ou un traitement d’une durée de 12 à 60 mois était indépendamment associé aux maladies abarticulaires. Le risque d’atteinte abarticulaire était également majoré par 4 pour un taux bas de LTCD4. Enfin, seul l’âge > 40 ans était associé risque d’arthrose. Conclusions. Près d’un tiers des PVV présentent des manifestations rhumatismales variées, en particulier ; les spondylarthrites, l’arthrose et les pathologies abarticulaires

Ultrasound Assessment of Bone Mass in Central Africans Population

Background: Osteodensitometry, a procedure increasingly accepted by clinicians to access osteoporosis is not yet fully validated by WHO. It requires the establishment of normal values and references curves for each population, and in our community, this is not yet assessed.The purpose of this study was twofold: to describe the profile of the speed of sound of proximal phalangeal metaphysis and establish references curves in central Africans and to compare them with those of other populations specially Caucasians.Methods: This cross sectional study was carried out at Kinshasa, Democratic Republic of the Congo in Central Africa from January to December 2016.Four hundred twenty four subjects aged from 20 to 87 years old, were explored and the speed of sound in theirs proximal phalanges measured using ultrasound equipment. Age, gender, hormonal status and speed of sound were collected and analyzed.Results: The mean value of the Amplitude Dependent Speed of Sound (AD-SoS) showed a growth feature with age (from 2056 m/sec at second decade of life to 2145.27 m/sec at fourth decade followed by a progressive decrease which was present in both sexes but more marked in postmenopausal women (1927.06 m/sec). This mean value was higher than in Caucasian studies. In this study, age and hormonal status seems to be the bone quality most influencing anthropometric factors as seen in many series.The curve profile with age was also a polynomial curve as seen in others populations of the world.Conclusion: This study provides normal data for phalangeal ultrasound measurements and reference curve of central Africans which were compared to those of Western studies

Ostéoporose et évaluation du risque fracturaire par l’outil frax chez des patients Congolais présentant un rhumatisme axial : Une série des cas multicentriques

Contexte et objectif. L’enjeu majeur dans le management de l’ostéoporose est l’identification des sujets à risque par la quantification du risque fracturaire. L’objectif de l’étude était d’évaluer le risque fracturaire chez les patients ayant consulté pour douleur du squelette axial. Méthodes. Il s’agissait d’une série des cas multicentriques menée sur des patients recrutés dans 8 hôpitaux de Kinshasa. Les paramètres d’intérêt comme l’âge, le sexe, l’alcoolisme, le tabagisme, la fracture de hanche chez un parent de 1er degré ou une fracture personnelle de fragilité ont été collectés auprès de chaque patient. La mesure de la densité osseuse avait été réalisée par absorptiométrie biphotonique à rayons X. Le risqué fracturaire a été évalué par le calcul de l’indice fracturaire FRAX. Ce risque était élevé lorsque la probabilité de survenue de fracture de hanche était ≥ 3% et/ou des fractures ostéoporotiques majeures ≥ 20%. Des tests statistiques usuels ont été utilisés pour l’analyse des résultats. Résultats. 90 patients dont 75 femmes étaient inclus. Leur âge moyen était de 63,5±12 ans. L’ostéoporose était diagnostiquée chez 34,4% des patients, l’ostéopénie chez 43,9% et 16,7% avaient une densité minérale osseuse normale. Aucune fracture ostéoporotique n’a été observée dans la présente étude, mais près de 30% de l’ensemble de l’échantillon avaient un risque fracturaire élevé. L’ostéoporose était associée, dans environ 80% des cas (p<0,005), à un risqué fracturaire élevé. Conclusion. La présente étude a montré que le risque fracturaire était élevé chez les patients atteints d’ostéoporose. Elle met en lumière la nécessité d’un dépistage précoce de cette pathologie. English title: Osteoporosis and assessment of fracturary risk using the frax tool in Congolese patients with axial rheumatism: A multicenter case series Context and objective. The major challenge in the management of osteoporosis is the identification of subjects at risk by quantifying the fracture risk in order to prevent the fracture cascade. The aim of the present study was to evaluate the fracture risk in patients who had consulted for axial skeletal pain. Methods. This was a multicenter case series carried out on patients with axial rheumatism recruited in 8 hospitals in Kinshasa. The parameters of interest such as age, sex, alcoholism, smoking, hip fracture in a 1st degree relative or personal fragility fracture were collected from each patient. Bone mineral density was measured by dual energy x-ray absorptiometry. Fracture risk was assessed by calculating the FRAX fracture index. This risk was considered high when the probability of occurrence of a hip fracture was ≥ 3% and/or major osteoporotic fractures ≥ 20%. Standard statistical tests were used to analyze the results. Results. 90 patients including 75 women (83.3%) were involved. Their average age was 63.5±12 years. Osteoporosis was diagnosed in 34.4% of patients, osteopenia in 43.9% of patients and 16.7% of patients had normal bone mineral density. No osteoporotic fractures were observed, but nearly 30% of the entire sample had a high fracture risk. Osteoporosis (T-score ≤-2.5) was associated, in approximately 80% of cases (p<0.005), with a high fracture risk. Conclusion. The present study showed that fracture risk was very high in patients with osteoporosis. It highlights the need for early detection of this pathology. Keywords: Osteoporosis, axial rheumatism, fracturary risk, FRA

Data from: Spondyloarthritis in the Democratic Republic of the Congo: a prospective hospital-based study

Abstract Objectives: To determine the spectrum of SpA in outpatients with rheumatic complaints attending two rheumatology practices in the Democratic Republic of Congo. Design: A descriptive prospective multicenter outpatient study. Setting: The present study analyzed 6 months data (from December 1st, 2012 till May 31st, 2013). Participants: 984 consecutive outpatients were studied. Interventions: none Primary and secondary outcome measures: A clinical diagnosis of SpA was made and several classification criteria were applied afterwards. Sacroiliac joint radiographic lesions were scored with the modified New York criteria. BASDAI and BASFI were evaluated in axial SpA. The primary end point was the prevalence of SpA and the secondary endpoints were the spectrum of SpA and its subtypes. Results: One hundred and five patients (10.7%) were diagnosed among 984 consecutive outpatients with a sex ratio (male to female) of 1.4. The average age at disease onset was 41.3±12.4 years. Non-radiographical axial spondyloarthritis was the most frequent subtype (5.0%) followed by reactive arthritis (4.3%). Other subtypes were: ankylosing spondylitis (1.0%), psoriatic arthritis (0.1%), SAPHO syndrome (0.1%) and IBD associated arthritis (0.1%). Mean BASDAI and BASFI in axial SpA were 42.7/100 and 46.4/100 respectively. Peripheral enthesitis was found in 43% of SpA patients and uveitis (10.4%) was the most frequent extra-articular manifestation. We did not detect any family history. Median erythrocyte sedimentation rate and C reactive protein were 37(range: 7-110) mm/h and 22 (range: 4-48) mg/l respectively. Conclusions: This hospital-based study suggests a substantial occurrence of some subtypes of SpA in central Africa. A population-based study is needed

Different subtypes of Spondyloarthritis in the University Hospital of Kinshasa and Provincial General Hospital of Kinshasa

Different subtypes of Spondyloarthritis in the University Hospital of Kinshasa and Provincial General Hospital of Kinshas

The phenotype and genotype of rheumatoid arthritis in the Democratic Republic of Congo

The aim of this study was to describe the phenotype and genotype of rheumatoid arthritis (RA) in Congolese patients.status: publishe

Prevalence and associated factors of subclinical atherosclerosis in rheumatoid arthritis at the university hospital of Kinshasa.

Background: Rheumatoid arthritis (RA) is associated with a 5 to 10 years reduction in life expectancy due to premature atherosclerosis. This reduction is the consequence of traditional cardiovascular risk factors (TCRF) as well as systemic inflammation. The aim of the present study was to describe the prevalence and factors associated with subclinical atherosclerosis in RA at the University Hospital of Kinshasa (UHK). Methods: Patients with a diagnosis of RA based on the 2010 ACR/EULAR criteria were included in this cross-sectional study from 1 June 2014 to 31 May 2015 at the UHK. RA disease activity was measured using the DAS28-ESR. Active RA was defined by a DAS 28 > 2.6. Severe RA was defined by the presence of extra-articular manifestation, joint erosions on X-rays or HAQ ≥0.5. An assessment of subclinical atherosclerosis was performed by the measurement of the carotid intima-media thickness (cIMT) using two-dimensional ultrasonography. Subclinical atherosclerosis was defined by a cIMT ≥0.9 mm. A diagnosis of atheroma plaque was retained when the cIMT was ≥1.5 mm. The association between subclinical atherosclerosis and potential risk factors was modeled using logistic regression analysis. Results: We recruited 75 patients. The average age was 51.8 ± 14.6 years, with a sex ratio F/M of 4. The prevalence of subclinical atherosclerosis was 32%. In logistic regression being a woman of ≥55 years old (aOR 10.6, 95% CI [2.087-53.82], p = 0.028), DAS28-ESR > 2.6 (aOR 3.5,95% CI [1.55-10.38], p = 0.044), severe RA (aOR 32.6,95% CI [1.761-60.37],p = 0.035), high blood pressure (aOR 22.4,95% CI [5.04-99.41], p = 0.005) and obesity (aOR 32.3, 95% CI [2.606-40.73], p = 0.026) emerged as factors associated with subclinical atherosclerosis. Conclusion: Subclinical atherosclerosis is common in RA patients attending the UHK. It appears to be associated with RA disease activity and severity apart from traditional cardiovascular risk factors. These results suggest that early management of subclinical atherosclerosis targeting remaining RA disease activity and cardiovascular risk factors could slow down progression to clinical cardiovascular disease.status: Published onlin